Wilson’s disease is the toxic effects from high level of copper in blood i.e., plasma, liver, brain, eyes and other organs.
Copper is a trace mineral nutrient required to help your body grow. Small amount of copper plays an important role in the development of healthy nerves, bones, collagen and the skin pigment melanin. It is primarily used for simple metabolic processes, for the development of the brain and central nervous system, for the formation of connective tissue that binds the parts of your body together and for building your bones etc.
Copper is not made by your body and must be taken in (absorbed) from your diet. With normal absorption and removal (excretion in faeces and urine), a healthy adult might have around 100 milligrams of copper in their body.
Normally, copper is absorbed from your food, and excess is excreted through a substance produced in your liver (bile).
The dietary intake of copper should be less than 1.0 mg per day.
Most people with Wilson’s disease are diagnosed between the ages of 5 and 35 but may effect at early age as well.
RISK FACTORS & CAUSES
It is a genetic / inherited disorder in which the liver is unable to excrete the metal copper and thereby it accumulates, in the system.
You can be at increased risk of Wilson’s disease if your parents or siblings have the condition.
Diagnosing the condition as early as possible dramatically increases the chances of successful treatment.
SIGN AND SYMPTOMS
- Liver swelling, albumin in urine, or water accumulation in abdominal cavities (ascites) or lower limbs.
- Copper accumulation in the brain cause neurological symptoms like tremors, sudden involuntary movements of the limbs rigidity and stiffness of joints.
- Difficulty in swallowing.
- Difficulty in movement of joints.
- Psychological disturbances like manic-depressive psychosis where patient has alternative states of over excitability and depression.
- Repeated abortions.
- Absence of menses.
- Fatigue, lack of appetite or abdominal pain
- A yellowing of the skin and the whites of the eye (jaundice)
- Golden-brown eye discoloration
- Problems with speech
- Clumsiness & loss of physical coordination issues
- Muscle stiffness
- Abdominal pain (in the ‘tummy’ or ‘belly’ area between the chest and pelvis)
- Vomiting blood (from bleeding varices).
- Contractions
- Distorted posture, twisting and repetitive gestures
- Slowness of movement (bradykinesia)
- Tremors in the arms, legs and head.
- A failing voice, slurring, drooling
- Loss of memory and mental confusion.
- Personality change
- Uncharacteristic, possibly bizarre behaviour
- Worry or anxiety (neurosis)
- Depression and mood swings
- Suicidal or homicidal thoughts
- Delusions or hallucinations (psychosis).
- Having a hard time falling asleep and staying asleep.
COMPLICATIONS
Untreated, Wilson’s disease can be fatal. Serious complications include:
- Scarring of the liver (cirrhosis).
- Liver failure.
- Persistent neurological problems.
- Kidney problems e.g., kidney stones and an abnormal number of amino acids excreted in the urine.
- Psychological problems e.g., personality changes, depression, irritability, bipolar disorder or psychosis.
- Blood problems e.g., destruction of red blood cells (haemolysis) leading to anaemia and jaundice.
DIETARY MANAGEMENT
- Avoid cooking in copper vessels.
- Avoid drinking alcohol. It can be harmful to the liver, if the liver has already be damaged from Wilson’s disease.
- Avoid foods that are high in copper: meat gelatin, lamb; pork; pheasant quail; duck; goose; squid; salmon; organ meats including liver, heart, kidney and brain.
- Sea plants / foods: shellfish, oysters, scallops, shrimp, lobster, clams, and crab.
- Soy and soy substitutes, nuts and seeds, beans, legumes; whole grain, wheat germ, almonds, broccoli, garlic; bran breads, cereals; fresh sweet potatoes.
- Dried beans including soybeans, lima beans, baked beans, garbanzo beans, pinto beans; dried peas; lentils; millet; barley.
- Vegetable juice, cocktail, mushrooms.
- Nectarine, commercially dried fruits including raisins, dates, prunes, avocado.
- Chocolate and some kind of beers are rich in copper.
CONSUME
- Increase intake of vitamin B6. Brewers yeast is very good source of vitamin B6.
- Meats, live, fish, eggs and milk, beans, nuts, legumes and whole grains, sunflower seeds, eggs, walnuts and herring.
- Kidney, liver, pork, poultry, salmon, wheat germ and avocado good source of vitamin B6 but are high in copper hence should be avoided.
- Read food labels, and check for copper content.
- Check labels of vitamin/mineral supplements to see if they contain copper.
- Analyse drinking water for copper content; if it contains more than 100 micrograms per litre, bottled demineralized water should be used. Water containing not more than 1 microgram of copper per litre can be consumed.
- Increase intake of zinc, it reduces copper levels:
- Plant foods are low in zinc, whole wheat, meat, poultry and milk, sea food.
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